Ewing sarcoma of the temporal bone with aneurysmal bone cyst-like changes: A rare case report with an unusual radiological presentation.

Ewing sarcoma (ES) is a malignant small round cell tumor, accounting for 10-15% of all primary bone tumors and approximately 3% of all pediatric cancers. Primary ES of the cranial bone is unusual with reported incidence from 1% to 6% of all ES cases. This report shows a rare case of primary ES of the squamous temporal bone in a 12-year-old boy with a history of swelling of the right temporal region and symptoms of increased intracranial pressure. We illustrate the extremely unusual radiological presentation of this primary ES of temporal bone associated with large aneurysmal bone cyst-like (ABC-like) changes. The boy was successfully treated according to Euro Ewing 2012 protocol. He is alive with no evidence of recurrence and metastasis after 16 months of completed treatment.

The benefit of complete resection of contrast enhancing tumor in glioblastoma patients: A population-based study.

Background: New treatment modalities have not been widely adopted for patients with glioblastoma (GBM) after the addition of temozolomide to radiotherapy. We hypothesize that increased extent of resection (EOR) has resulted in improved survival for surgically treated patients with glioblastoma at the population level. Methods: Retrospective analysis of adult patients operated for glioblastoma in the population of South-Eastern Norway. Patients were stratified into Pre-temozolomide- (2003-2005), temozolomide- (2006-2012), and resection-focused period (2013-2019) and evaluated according to age and EOR. Results: The study included 1657 adult patients operated on for supratentorial glioblastoma. The incidence of histologically confirmed glioblastoma increased from 3.7 in 2003 to 5.3 per 100 000 in 2019. The median survival was 11.4 months. Complete resection of contrast-enhancing tumor (CRCET) was achieved in 386 patients, and this fraction increased from 13% to 32% across the periods. Significant improvement in median survival was found between the first 2 periods and the last (10.5 and 10.6 vs. 12.3 months; P < .01), with a significant increase in 3- and 5-year survival probability to 12% and 6% (P < .01). Patients with CRCET survived longer than patients with non-CRCET (16.1 vs. 10.8 months; P < .001). The median survival doubled in patients ≥70 years and (12.1 months). Survival was similar between the time periods in patients where CRCET was achieved. Conclusions: We demonstrate an improved survival of GBM patients at the population level associated with an increased fraction of patients with CRCET. The data support the importance of CRCET to improve glioblastoma patient outcomes.

Diagnostic utility of Restriction Spectrum Imaging in the characterization of the peritumoral brain zone in glioblastoma: Analysis of overall and progression-free survival.

PURPOSE: We studied the ability of Restriction Spectrum Imaging (RSI), a novel advanced diffusion imaging technique, to estimate levels of cellularity in different glioblastoma regions, evaluated their prognostic value compared with established clinical diffusion metrics such as fractional anisotropy (FA) and mean diffusivity (MD). METHODS: Forty-two patients with untreated glioblastoma, IDH-wildtype, were examined with an advanced MRI tumor protocol. The region of interest (ROI) was obtained from the contrast-enhancing part of tumor and the peritumoral brain zones and then co-registered with RSI-cellularity index, FA and MD maps. Histogram parameters of diffusion metrics were assessed for all ROI locations and compared to MGMT promoter methylation status and survival. The ability of RSI-cellularity index, FA, and MD to stratify survival and were assessed by Cox proportional hazard regression, adjusted for significant clinical predictors. RESULTS: The highest RSI-cellularity index was measured in contrast-enhancing tumor core with a negative gradient from tumor core to the periphery of peritumoral zone with predictive accuracy 81 % (P < 0.001). Shorter overall survival was significant associated with higher RSI-cellularity index (hazard ratio (HR) 3.6, 95 % confidence interval (CI) 1.3-9.5, P = 0.002) with synchronal decrease in MD (HR 0.31, 95 %CI 0.1-0.8, P = 0.008) in the contrast-enhanced tumor core. This association was also consistent for RSI-cellularity index value measured in the peri-enhancing zone (HR 3.6, 95 % CI 1.0-12.3, P = 0.041). No statistically significant differences were noted between RSI-cellularity index, FA, nor MD and MGMT promoter methylation. CONCLUSION: RSI-cellularity index may be used as prognostic biomarker to improve risk stratification in patients with glioblastoma.

Sudden death in epilepsy and ectopic neurohypophysis in Joubert syndrome 23 diagnosed using SNVs/indels and structural variants pipelines on WGS data: a case report.

BACKGROUND: Joubert syndrome (JBTS) is a genetically heterogeneous group of neurodevelopmental syndromes caused by primary cilia dysfunction. Usually the neurological presentation starts with abnormal neonatal breathing followed by muscular hypotonia, psychomotor delay, and cerebellar ataxia. Cerebral MRI shows mid- and hindbrain anomalies including the molar tooth sign. We report a male patient with atypical presentation of Joubert syndrome type 23, thus expanding the phenotype. CASE PRESENTATION: Clinical features were consistent with JBTS already from infancy, yet the syndrome was not suspected before cerebral MRI later in childhood showed the characteristic molar tooth sign and ectopic neurohypophysis. From age 11 years seizures developed and after few years became increasingly difficult to treat, also related to inadequate compliance to therapy. He died at 23 years of sudden unexpected death in epilepsy (SUDEP). The genetic diagnosis remained elusive for many years, despite extensive genetic testing. We reached the genetic diagnosis by performing whole genome sequencing of the family trio and analyzing the data with the combination of one analysis pipeline for single nucleotide variants (SNVs)/indels and one for structural variants (SVs). This lead to the identification of the most common variant detected in patients with JBTS23 (OMIM# 616490), rs534542684, in compound heterozygosity with a 8.3 kb deletion in KIAA0586, not previously reported. CONCLUSIONS: We describe for the first time ectopic neurohypophysis and SUDEP in JBTS23, expanding the phenotype of this condition and raising the attention on the possible severity of the epilepsy in this disease. We also highlight the diagnostic power of WGS, which efficiently detects SNVs/indels and in addition allows the identification of SVs.

Dynamic susceptibility contrast and diffusion MR imaging identify oligodendroglioma as defined by the 2016 WHO classification for brain tumors: histogram analysis approach.

PURPOSE: According to the revised World Health Organization (WHO) Classification of Tumors of the Central Nervous System (CNS) of 2016, oligodendrogliomas are now defined primarily by a specific molecular signature (presence of IDH mutation and 1p19q codeletion). The purpose of our study was to assess the value of dynamic susceptibility contrast MR imaging (DSC-MRI) and diffusion-weighted imaging (DWI) to characterize oligodendrogliomas and to distinguish them from astrocytomas. METHODS: Seventy-one adult patients with untreated WHO grade II and grade III diffuse infiltrating gliomas and known 1p/19q codeletion status were retrospectively identified and analyzed using relative cerebral blood volume (rCBV) and apparent diffusion coefficient (ADC) maps based on whole-tumor volume histograms. The Mann-Whitney U test and logistic regression were used to assess the ability of rCBV and ADC to differentiate between oligodendrogliomas and astrocytomas both independently, but also related to the WHO grade. Prediction performance was evaluated in leave-one-out cross-validation (LOOCV). RESULTS: Oligodendrogliomas showed significantly higher microvascularity (higher rCBVMean ≥ 0.80, p = 0.013) and higher vascular heterogeneity (lower rCBVPeak ≤ 0.044, p = 0.015) than astrocytomas. Diffuse gliomas with higher cellular density (lower ADCMean ≤ 1094 × 10-6 mm2/s, p = 0.009) were more likely to be oligodendrogliomas than astrocytomas. Histogram analysis of rCBV and ADC was able to differentiate between diffuse astrocytomas (WHO grade II) and anaplastic astrocytomas (WHO grade III). CONCLUSION: Histogram-derived rCBV and ADC parameter may be used as biomarkers for identification of oligodendrogliomas and may help characterize diffuse gliomas based upon their genetic characteristics.

Texture analysis on diffusion tensor imaging: discriminating glioblastoma from single brain metastasis.

BACKGROUND: Texture analysis has been done on several radiological modalities to stage, differentiate, and predict prognosis in many oncologic tumors. PURPOSE: To determine the diagnostic accuracy of discriminating glioblastoma (GBM) from single brain metastasis (MET) by assessing the heterogeneity of both the solid tumor and the peritumoral edema with magnetic resonance imaging (MRI) texture analysis (MRTA). MATERIAL AND METHODS: Preoperative MRI examinations done on a 3-T scanner of 43 patients were included: 22 GBM and 21 MET. MRTA was performed on diffusion tensor imaging (DTI) in a representative region of interest (ROI). The MRTA was assessed using a commercially available research software program (TexRAD) which applies a filtration histogram technique for characterizing tumor and peritumoral heterogeneity. The filtration step selectively filters and extracts texture features at different anatomical scales varying from 2 mm (fine) to 6 mm (coarse). Heterogeneity quantification was obtained by the statistical parameter entropy. A threshold value to differentiate GBM from MET with sensitivity and specificity was calculated by receiver operating characteristic (ROC) analysis. RESULTS: Quantifying the heterogeneity of the solid part of the tumor showed no significant difference between GBM and MET. However, the heterogeneity of the GBMs peritumoral edema was significantly higher than the edema surrounding MET, differentiating them with a sensitivity of 80% and specificity of 90%. CONCLUSION: Assessing the peritumoral heterogeneity can increase the radiological diagnostic accuracy when discriminating GBM and MET. This will facilitate the medical staging and optimize the planning for surgical resection of the tumor and postoperative management.

Survival Associations Using Perfusion and Diffusion Magnetic Resonance Imaging in Patients With Histologic and Genetic Defined Diffuse Glioma World Health Organization Grades II and III.

OBJECTIVE: According to the new World Health Organization 2016 classification for tumors of the central nervous system, 1p/19q codeletion defines the genetic hallmark that differentiates oligodendrogliomas from diffuse astrocytomas. The aim of our study was to evaluate whether relative cerebral blood volume (rCBV) and apparent diffusion coefficient (ADC) histogram analysis can stratify survival in adult patients with genetic defined diffuse glioma grades II and III. METHODS: Sixty-seven patients with untreated diffuse gliomas World Health Organization grades II and III and known 1p/19q codeletion status were included retrospectively and analyzed using ADC and rCBV maps based on whole-tumor volume histograms. Overall survival and progression-free survival (PFS) were analyzed by using Kaplan-Meier and Cox survival analyses adjusted for known survival predictors. RESULTS: Significant longer PFS was associated with homogeneous rCBV distribution-higher rCBVpeak (median, 37 vs 26 months; hazard ratio [HR], 3.2; P = 0.02) in patients with astrocytomas, and heterogeneous rCBV distribution-lower rCBVpeak (median, 46 vs 37 months; HR, 5.3; P < 0.001) and higher rCBVmean (median, 44 vs 39 months; HR, 7.9; P = 0.003) in patients with oligodendrogliomas. Apparent diffusion coefficient parameters (ADCpeak, ADCmean) did not stratify PFS and overall survival. CONCLUSIONS: Tumors with heterogeneous perfusion signatures and high average values were associated with longer PFS in patients with oligodendrogliomas. On the contrary, heterogeneous perfusion distribution was associated with poor outcome in patients with diffuse astrocytomas.

Patterns of Invasive Growth in Malignant Gliomas-The Hippocampus Emerges as an Invasion-Spared Brain Region.

BACKGROUND: Widespread infiltration of tumor cells into surrounding brain parenchyma is a hallmark of malignant gliomas, but little data exist on the overall invasion pattern of tumor cells throughout the brain. METHODS: We have studied the invasive phenotype of malignant gliomas in two invasive mouse models and patients. Tumor invasion patterns were characterized in a patient-derived xenograft mouse model using brain-wide histological analysis and magnetic resonance (MR) imaging. Findings were histologically validated in a cdkn2a-/- PDGF-ß lentivirus-induced mouse glioblastoma model. Clinical verification of the results was obtained by analysis of MR images of malignant gliomas. RESULTS: Histological analysis using human-specific cellular markers revealed invasive tumors with a non-radial invasion pattern. Tumors cells accumulated in structures located far from the transplant site, such as the optic white matter and pons, whereas certain adjacent regions were spared. As such, the hippocampus was remarkably free of infiltrating tumor cells despite the extensive invasion of surrounding regions. Similarly, MR images of xenografted mouse brains displayed tumors with bihemispheric pathology, while the hippocampi appeared relatively normal. In patients, most malignant temporal lobe gliomas were located lateral to the collateral sulcus. Despite widespread pathological fluid-attenuated inversion recovery signal in the temporal lobe, 74% of the "lateral tumors" did not show signs of involvement of the amygdalo-hippocampal complex. CONCLUSIONS: Our data provide clear evidence for a compartmental pattern of invasive growth in malignant gliomas. The observed invasion patterns suggest the presence of preferred migratory paths, as well as intra-parenchymal boundaries that may be difficult for glioma cells to traverse supporting the notion of compartmental growth. In both mice and human patients, the hippocampus appears to be a brain region that is less prone to tumor invasion.

Imaging spectrum of central nervous system complications of hematopoietic stem cell and solid organ transplantation.

Neurologic complications are common after hematopoietic stem cell transplantation (HSCT) and solid organ transplantation (SOT) and affect 30-60% of transplant recipients. The aim of this article is to provide a practical imaging approach based on the timeline and etiology of CNS abnormalities, and neurologic complications related to transplantation of specific organs. The lesions will be classified based upon the interval from HSCT procedure: pre-engraftment period <30 days, early post-engraftment period 30-100 days, late post-engraftment period >100 days, and the interval from SOT procedure: postoperative phase 1-4 weeks, early posttransplant syndromes 1-6 months, late posttransplant syndromes >6 months. Further differentiation will be based on etiology: infections, drug toxicity, metabolic derangements, cerebrovascular complications, and posttransplantation malignancies. In addition, differentiation will be based on complications specific to the type of transplantation: allogeneic and autologous hematopoietic stem cells (HSC), heart, lung, kidney, pancreas, and liver. Thus, in this article we emphasize the strategic role of neuroradiology in the diagnosis and response to treatment by utilizing a methodical approach in the work up of patients with neurologic complications after transplantation.

Diagnostic performance of texture analysis on MRI in grading cerebral gliomas.

BACKGROUND AND PURPOSE: Grading of cerebral gliomas is important both in treatment decision and assessment of prognosis. The purpose of this study was to determine the diagnostic accuracy of grading cerebral gliomas by assessing the tumor heterogeneity using MRI texture analysis (MRTA). MATERIAL AND METHODS: 95 patients with gliomas were included, 27 low grade gliomas (LGG) all grade II and 68 high grade gliomas (HGG) (grade III=34 and grade IV=34). Preoperative MRI examinations were performed using a 3T scanner and MRTA was done on preoperative contrast-enhanced three-dimensional isotropic spoiled gradient echo images in a representative ROI. The MRTA was assessed using a commercially available research software program (TexRAD) that applies a filtration-histogram technique for characterizing tumor heterogeneity. Filtration step selectively filters and extracts texture features at different anatomical scales varying from 2mm (fine features) to 6mm (coarse features), the statistical parameter standard deviation (SD) was obtained. Receiver operating characteristics (ROC) was performed to assess sensitivity and specificity for differentiating between the different grades and calculating a threshold value to quantify the heterogeneity. RESULTS: LGG and HGG was best discriminated using SD at fine texture scale, with a sensitivity and specificity of 93% and 81% (AUC 0.910, p<0.0001). The diagnostic ability for MRTA to differentiate between the different sub-groups (grade II-IV) was slightly lower but still significant. CONCLUSIONS: Measuring heterogeneity in gliomas to discriminate HGG from LGG and between different histological sub-types on already obtained images using MRTA can be a useful tool to augment the diagnostic accuracy in grading cerebral gliomas and potentially hasten treatment decision.

Volumetric glioma quantification: comparison of manual and semi-automatic tumor segmentation for the quantification of tumor growth.

BACKGROUND: Volumetric magnetic resonance imaging (MRI) is now widely available and routinely used in the evaluation of high-grade gliomas (HGGs). Ideally, volumetric measurements should be included in this evaluation. However, manual tumor segmentation is time-consuming and suffers from inter-observer variability. Thus, tools for semi-automatic tumor segmentation are needed. PURPOSE: To present a semi-automatic method (SAM) for segmentation of HGGs and to compare this method with manual segmentation performed by experts. The inter-observer variability among experts manually segmenting HGGs using volumetric MRIs was also examined. MATERIAL AND METHODS: Twenty patients with HGGs were included. All patients underwent surgical resection prior to inclusion. Each patient underwent several MRI examinations during and after adjuvant chemoradiation therapy. Three experts performed manual segmentation. The results of tumor segmentation by the experts and by the SAM were compared using Dice coefficients and kappa statistics. RESULTS: A relatively close agreement was seen among two of the experts and the SAM, while the third expert disagreed considerably with the other experts and the SAM. An important reason for this disagreement was a different interpretation of contrast enhancement as either surgically-induced or glioma-induced. The time required for manual tumor segmentation was an average of 16 min per scan. Editing of the tumor masks produced by the SAM required an average of less than 2 min per sample. CONCLUSION: Manual segmentation of HGG is very time-consuming and using the SAM could increase the efficiency of this process. However, the accuracy of the SAM ultimately depends on the expert doing the editing. Our study confirmed a considerable inter-observer variability among experts defining tumor volume from volumetric MRIs.

Analysis of diffusion tensor imaging metrics for gliomas grading at 3 T.

OBJECTIVES: To assess the diagnostic accuracy of axial diffusivity (AD), radial diffusivity (RD), apparent diffusion coefficient (ADC) and fractional anisotropy (FA) values derived from DTI for grading of glial tumors, and to estimate the correlation between DTI parameters and tumor grades. METHODS: Seventy-eight patients with glial tumors underwent DTI. AD, RD, ADC and FA values of tumor, peritumoral edema and contralateral normal-appearing white matter (NAWM) and AD, RD, ADC and FA ratios: lowest average AD, RD, ADC and FA values in tumor or peritumoral edema to AD, RD, ADC and FA of NAWM were calculated. DTI parameters and tumor grades were analyzed statistically and with Pearson correlation. Receiver operating characteristic (ROC) curve analysis was also performed. RESULTS: The differences in ADC, AD and RD tumor values, and ADC and RD tumor ratios were statistically significant between grades II and III, grades II and IV, and between grades II and III-IV. The AD tumor ratio differed significantly among all tumor grades. Tumor ADC, AD, RD and glial tumor grades were strongly correlated. In the ROC curve analysis, the area under the curve (AUC) of the parameter tumor ADC was the largest for distinguishing grade II from grades III to IV (98.5%), grade II from grade IV (98.9%) and grade II from grade III (97.0%). CONCLUSION: ADC, RD and AD are useful DTI parameters for differentiation between low- and high-grade gliomas with a diagnostic accuracy of more than 90%. Our study revealed a good inverse correlation between ADC, RD, AD and WHO grades II-IV astrocytic tumors.

Measurements of diagnostic examination performance and correlation analysis using microvascular leakage, cerebral blood volume, and blood flow derived from 3T dynamic susceptibility-weighted contrast-enhanced perfusion MR imaging in glial tumor grading.

INTRODUCTION: To assess the diagnostic accuracy of microvascular leakage (MVL), cerebral blood volume (CBV) and blood flow (CBF) values derived from dynamic susceptibility-weighted contrast-enhanced perfusion MR imaging (DSC-MR imaging) for grading of cerebral glial tumors, and to estimate the correlation between vascular permeability/perfusion parameters and tumor grades. METHODS: A prospective study of 79 patients with cerebral glial tumors underwent DSC-MR imaging. Normalized relative CBV (rCBV) and relative CBF (rCBF) from tumoral (rCBVt and rCBFt), peri-enhancing region (rCBVe and rCBFe), and the value in the tumor divided by the value in the peri-enhancing region (rCBVt/e and rCBFt/e), as well as MVL, expressed as the leakage coefficient K(2) were calculated. Hemodynamic variables and tumor grades were analyzed statistically and with Pearson correlations. Receiver operating characteristic (ROC) curve analyses were also performed for each of the variables. RESULTS: The differences in rCBVt and the maximum MVL (MVL(max)) values were statistically significant among all tumor grades. Correlation analysis using Pearson was as follows: rCBVt and tumor grade, r = 0.774; rCBFt and tumor grade, r = 0.417; MVL(max) and tumor grade, r = 0.559; MVL(max) and rCBVt, r = 0.440; MVL(max) and rCBFt, r = 0.192; and rCBVt and rCBFt, r = 0.605. According to ROC analyses for distinguishing tumor grade, rCBVt showed the largest areas under ROC curve (AUC), except for grade III from IV. CONCLUSION: Both rCBVt and MVL(max) showed good discriminative power in distinguishing all tumor grades. rCBVt correlated strongly with tumor grade; the correlation between MVL(max) and tumor grade was moderate.

Measurements of diagnostic examination performance using quantitative apparent diffusion coefficient and proton MR spectroscopic imaging in the preoperative evaluation of tumor grade in cerebral gliomas.

PURPOSE: Tumor grading is very important both in treatment decision and evaluation of prognosis. While tissue samples are obtained as part of most therapeutic approaches, factors that may result in inaccurate grading due to sampling error (namely, heterogeneity in tissue sampling, as well as tumor-grade heterogeneity within the same tumor specimen), have led to a desire to use imaging better to ascertain tumor grade. The purpose in our study was to evaluate the sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), area under the curve (AUC), and accuracy of diffusion-weighted MR imaging (DWI), proton MR spectroscopic imaging (MRSI) or both in grading primary cerebral gliomas. MATERIALS AND METHODS: We performed conventional MR imaging (MR), DWI, and MRSI in 74 patients with newly diagnosed brain gliomas: 59 patients had histologically verified high-grade gliomas: 37 glioblastomas multiform (GBM) and 22 anaplastic astrocytomas (AA), and 15 patients had low-grade gliomas. Apparent diffusion coefficient (ADC) values of tumor and peritumoral edema, and ADC ratios (ADC in tumor or peritumoral edema to ADC of contralateral white matter, as well as ADC in tumor to ADC in peritumoral edema) were determined from three regions of interest. The average of the mean, maximum, and minimum for ADC variables was calculated for each patient. The metabolite ratios of Cho/Cr and Cho/NAA at intermediate TE were assessed from spectral maps in the solid portion of tumor, peritumoral edema and contralateral normal-appearing white matter. Tumor grade determined with the two methods was then compared with that from histopathologic grading. Logistic regression and receiver operating characteristic (ROC) curve analysis were performed to determine optimum thresholds for tumor grading. Measures of diagnostic examination performance, such as sensitivity, specificity, PPV, NPV, AUC, and accuracy for identifying high-grade gliomas were also calculated. RESULTS: Statistical analysis demonstrated a threshold minimum ADC tumor value of 1.07 to provide sensitivity, specificity, PPV, and NPV of 79.7%, 60.0%, 88.7%, and 42.9% respectively, in determining high-grade gliomas. Threshold values of 1.35 and 1.78 for peritumoral Cho/Cr and Cho/NAA metabolite ratios resulted in sensitivity, specificity, PPV, and NPV of 83.3%, 85.1%, 41.7%, 97.6%, and 100%, 57.4%, 23.1% and 100% respectively for determining high-grade gliomas. Significant differences were noted in the ADC tumor values and ratios, peritumoral Cho/Cr and Cho/NAA metabolite ratios, and tumoral Cho/NAA ratio between low- and high-grade gliomas. The combination of mean ADC tumor value, maximum ADC tumor ratio, peritumoral Cho/Cr and Cho/NAA metabolite ratios resulted in sensitivity, specificity, PPV, and NPV of 91.5%, 100%, 100% and 60% respectively. CONCLUSION: Combining DWI and MRSI increases the accuracy of preoperative imaging in the determination of glioma grade. MRSI had superior diagnostic performance in predicting glioma grade compared with DWI alone. The predictive values are helpful in the clinical decision-making process to evaluate the histologic grade of tumors, and provide a means of guiding treatment.

Diagnostic examination performance by using microvascular leakage, cerebral blood volume, and blood flow derived from 3-T dynamic susceptibility-weighted contrast-enhanced perfusion MR imaging in the differentiation of glioblastoma multiforme and brain metastasis.

INTRODUCTION: Conventional magnetic resonance (MR) imaging has limited capacity to differentiate between glioblastoma multiforme (GBM) and metastasis. The purposes of this study were: (1) to compare microvascular leakage (MVL), cerebral blood volume (CBV), and blood flow (CBF) in the distinction of metastasis from GBM using dynamic susceptibility-weighted contrast-enhanced perfusion MR imaging (DSC-MRI), and (2) to estimate the diagnostic accuracy of perfusion and permeability MR imaging. METHODS: A prospective study of 61 patients (40 GBMs and 21 metastases) was performed at 3 T using DSC-MRI. Normalized rCBV and rCBF from tumoral (rCBVt, rCBFt), peri-enhancing region (rCBVe, rCBFe), and by dividing the value in the tumor by the value in the peri-enhancing region (rCBVt/e, rCBFt/e), as well as MVL were calculated. Hemodynamic and histopathologic variables were analyzed statistically and Spearman/Pearson correlations. Receiver operating characteristic curve analysis was performed for each of the variables. RESULTS: The rCBVe, rCBFe, and MVL were significantly greater in GBMs compared with those of metastases. The optimal cutoff value for differentiating GBM from metastasis was 0.80 which implies a sensitivity of 95%, a specificity of 92%, a positive predictive value of 86%, and a negative predictive value of 97% for rCBVe ratio. We found a modest correlation between rCBVt and rCBFt ratios. CONCLUSION: MVL measurements in GBMs are significantly higher than those in metastases. Statistically, both rCBVe, rCBVt/e and rCBFe, rCBFt/e were useful in differentiating between GBMs and metastases, supporting the hypothesis that perfusion MR imaging can detect infiltration of tumor cells in the peri-enhancing region.

Proton magnetic resonance spectroscopy in the distinction of high-grade cerebral gliomas from single metastatic brain tumors.

BACKGROUND: Brain metastases and primary high-grade gliomas, including glioblastomas multiforme (GBM) and anaplastic astrocytomas (AA), may be indistinguishable by conventional magnetic resonance (MR) imaging. Identification of these tumors may have therapeutic consequences. PURPOSE: To assess the value of MR spectroscopy (MRS) using short and intermediate echo time (TE) in differentiating solitary brain metastases and high-grade gliomas on the basis of differences in metabolite ratios in the intratumoral and peritumoral region. MATERIAL AND METHODS: We performed MR imaging and MRS in 73 patients with histologically verified intraaxial brain tumors: 53 patients with high-grade gliomas (34 GBM and 19 AA) and 20 patients with metastatic brain tumors. The metabolite ratios of Cho/Cr, Cho/NAA, and NAA/Cr at intermediate TE and the presence of lipids at short TE were assessed from spectral maps in the tumoral core, peritumoral edema, and contralateral normal-appearing white matter. The differences in the metabolite ratios between high-grade gliomas/GBM/AA and metastases were analyzed statistically. Cutoff values of Cho/Cr, Cho/NAA, and NAA/Cr ratios in the peritumoral edema, as well as Cho/Cr and NAA/Cr ratios in the tumoral core for distinguishing high-grade gliomas/GBM/AA from metastases were determined by receiver operating characteristic (ROC) curve analysis. RESULTS: Significant differences were noted in the peritumoral Cho/Cr, Cho/NAA, and NAA/ Cr ratios between high-grade gliomas/GBM/AA and metastases. ROC analysis demonstrated a cutoff value of 1.24 for peritumoral Cho/Cr ratio to provide sensitivity, specificity, positive (PPV), and negative predictive values (NPV) of 100%, 88.9%, 80.0%, and 100%, respectively, for discrimination between high-grade gliomas and metastases. By using a cutoff value of 1.11 for peritumoral Cho/NAA ratio, the sensitivity was 100%, the specificity was 91.1%, the PPV was 83.3%, and the NPV was 100%. CONCLUSION: The results of this study demonstrate that MRS can differentiate high-grade gliomas from metastases, especially with peritumoral measurements, supporting the hypothesis that MRS can detect infiltration of tumor cells in the peritumoral edema.

[Epilepsia partialis continua (Kojevnikov's syndrome)]. / Epilepsia partialis continua (Kojevnikovs syndrom).

BACKGROUND: Lesions close to the central sulcus may give rise to focal motor seizures of long duration. This condition is called epilepsia partialis continua (Kojevnikov's syndrome). MATERIAL AND METHODS: Over the last two years, the National Centre for Epilepsy in Norway has treated 12 patients with epilepsia partialis continua. We discuss the occurrence, etiologies, semiology, findings from supplementary investigations, and therapeutic options on the basis of relevant literature and our own experience with these patients. RESULTS AND INTERPRETATION: Morphological lesions were found in 10 out of these 12 patients; cortical dysplasia in 3 patients, brain tumour in 2 patients, cerebral infarction in 2 patients, Rasmussen syndrome in 2 patients, and cerebral haemorrhage from an arteriovenous malformation in 1 patient. 9 patients had intermittent periods of jerking lasting from some hours to several days; the remaining 3 had permanent jerks. One of them had had this condition for 44 years. In 11 patients the jerks were localised to the face and/or the hand. The effect of antiepileptic drugs was disappointing; none became seizure-free. Five patients had undergone surgery. Surgical lesionectomy in this brain area is associated with a high risk of damage to eloquent cortex, but multiple subpial transections may have a seizure-blocking effect. One patient with Rasmussen's syndrome became seizure-free after a functional hemispherotomy.

A patient with a 44-year history of epilepsia partialis continua caused by a perirolandic cortical dysplasia.

Epilepsia partialis continua (EPC), or Kojevnikov's syndrome, is a rare epileptic syndrome arising from a variety of lesions in the perirolandic area. We report herein a 46-year-old woman with drug-resistant EPC due to a cortical dysplasia in the left frontoparietal region. For 44 years she has suffered continuous right-sided jerks, particularly in the right arm and hand, with an average frequency of 10-20 jerks per minute. During EEG recordings her jerks were associated with spikes and sharp waves over the left frontocentroparietal region, sometimes also with bursts of high-voltage generalized spike-wave complexes with a maximum bicentrally, followed by an electrodecrement. Despite the continuous jerks she is independent in daily life activities, and she considers the jerks not severe enough to justify surgery, i.e., multiple subpial transections.